Published on 18/03/2015 by admin
Filed under Dermatology
Last modified 18/03/2015
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John Berth-Jones
Evidence Levels: A Double-blind study B Clinical trial ≥ 20 subjects C Clinical trial < 20 subjects D Series ≥ 5 subjects E Anecdotal case reports
Pyoderma gangrenosum (PG) is a clinically diagnosed entity presenting as pustules which enlarge, forming ulcers with a dark, necrotic, undermined margin. Any skin site may be affected. Although PG is usually self-limiting there is a danger of disfiguring scarring.
No treatment for PG is always effective. The most consistent results are reported with systemic corticosteroids and cyclosporine, and these effective but potentially toxic modalities can be employed when the severity of the disease justifies the risks (e.g., when there is facial involvement or rapid progression). Infliximab has been shown to be beneficial in a controlled trial. The evidence regarding other modalities is less conclusive. PG tends to resolve spontaneously, so some of the reports of therapeutic success may simply be the result of the disease following its natural course.
In cases where scarring is not a major concern, consideration should be given to conservative treatment with wound dressing only, as most lesions resolve spontaneously. When required, relatively non-toxic first-line treatments should be tried first and second-line modalities employed if there is no response. Topical tacrolimus offers an interesting novel approach to first-line therapy. A wide variety of potentially hazardous and expensive treatments are employed occasionally, and these third-line modalities should be considered only when others are ineffective.
The many recognized associations with PG include rheumatoid disease, inflammatory bowel disease, myeloproliferative disease such as acute myeloblastic leukemia, plasma cell dyscrasias, and Wegener’s granulomatosis. These may warrant investigation, and may also influence the choice of treatment. Effective management of an underlying pathology such as ulcerative colitis often seems to result in improvement of the PG.
PG may demonstrate the Koebner phenomenon; care should be taken to avoid trauma to the skin. When surgery is unavoidable in patients with a history of PG, the surgeon should be made aware of this risk. Surgical incisions should be kept as short as possible. Careful wound closure may be helpful. Prophylactic systemic corticoids or cyclosporine may be indicated perioperatively.
Hematology
Plasma protein electrophoresis
Rheumatoid factor
ANCA
In selected patients where clinically indicated:
Gastrointestinal work-up for inflammatory bowel disease
Reich K, Vente C, Neumann C. Br J Dermatol 1998; 139: 755–7.
Tacrolimus ointment 0.1% proved effective when used in combination with systemic cyclosporine, and also when used alone. The formula comprised 100 mg FK506 powder obtained from tablets of Prograf, mixed into 100 g of hydrophilic petrolatum (white petrolatum with 8% bleached beeswax, 3% stearyl alcohol, and 3% cholesterol).
Lyon CC, Stapleton M, Smith AJ, Mendelsohn S, Beck MH, Griffiths CEM. J Dermatol Treat 2001; 12: 13–17.
Seven out of 11 cases of peristomal PG healed completely after 2 to 10 weeks of applying tacrolimus 0.3% in carmellose sodium paste (Orabase). Serum levels of tacrolimus were undetectable in all cases. In this open study the results were at least as good as those from clobetasol propionate.
Peachey RDG. Br J Dermatol 1974; 90: 106.
A case of PG involving the scalp showed slow but steady improvement on treatment with 0.1% betamethasone valerate lotion applied under polythene occlusion at night.
Chriba M, Skellett AM, Levell NJ. Clin Exp Dermatol 2010; 35: 337–8.
A case of peristomal PG healed over 4 weeks using beclomethasone dipropionate 200 µg, four puffs daily.
The accepted efficacy of systemic corticosteroids would suggest that topical application might also be beneficial. However, there are only sparse anecdotal reports to support their efficacy. Potent compounds are generally used, applied once daily under an occlusive dressing.
Lorincz AL, Pearson RW. Arch Dermatol 1962; 85: 42–56.
Dapsone was successfully employed in the treatment of PG at doses of up to 400 mg daily. Low cost and the familiarity of dermatologists with this drug probably contribute to its popularity.
Dapsone has often been used in combination with other modalities, especially systemic corticosteroids. There are also many published cases in which this drug has proved ineffective. Dapsone has been successfully used in children with PG. The mechanism of action is believed to be inhibition of neutrophil migration and the myeloperoxidase system. Crushed dapsone applied topically is reported as effective in a case of peristomal PG.
Gardner LW, Acker DW. Arch Dermatol 1972; 106: 599–600.
A case of multifocal PG responded to intralesional triamcinolone acetonide 10 mg/mL. Doses ranging from 40 mg to–200 mg were injected at any one time.
Intralesional or perilesional injection of corticosteroids appears to be very helpful in some cases. The corticosteroid is usually injected into the skin around the active margins of lesions.
Berth-Jones J, Tan SV, Graham-Brown RAC, Pembroke AC. J Dermatol Treat 1989; 1: 23–5.
Seven cases responded to minocycline at doses of 100 mg twice daily or 200 mg twice daily. Improvement was often observed within a few days.
Patel GK, Rhodes JR, Evans B, Holt PJ. J Dermatol Treat 2004; 15: 122–5.
Two cases responded to topical application of 0.5% nicotine in cetamacrogol cream.
There are also reports of PG responding to nicotine chewing gum, application of nicotine patches to lesions, and application of Swedish moist snuff containing nicotine.
Bellini V, Simonetti S, Lisi P. J Eur Acad Dermatol Venereol 2008; 22: 113–15.
A patient was cleared of PG lesions within 8 weeks using pimecrolimus cream twice daily.
Perry HO, Brunsting LA. Arch Dermatol 1957; 75: 380–6.
Six out of seven patients with PG associated with colitis demonstrated a good response to sulfasalazine 0.5 g every 3 hours. A good response was also seen in three of four cases of PG when colitis was not present.
This drug may be particularly useful in cases of PG associated with inflammatory bowel disease, and it can also be effective in those which are not. The initial dose ranges from 0.5–2 g four times daily. Doses at the upper end of this range are usually reduced for maintenance therapy.
De Cock KM, Thorne MG. Br J Dermatol 1980; 102: 231–3.
Two cases responded to sodium cromoglycate aqueous solution (2% w/v, Rynacrom nasal spray). In one patient, healing occurred within 3 weeks.
This is a remarkably safe treatment. Solutions of this drug have been applied to lesions of PG in concentrations ranging from 1% to 4%. Various nasal sprays and nebulizer solutions have proved suitable for direct application to PG lesions. The solution can be sprayed on to the ulcer, or applied on gauze or under occlusion with a hydrocolloid dressing. This drug may act by inhibiting neutrophil migration or cytotoxicity.
Elgart G, Stover P, Larson K, Sutter C, Scheibner S, Davis B, et al. J Am Acad Dermatol 1991; 24: 83–6.
Six of seven patients, including cases associated with rheumatoid disease and cryoglobulinemia, improved on cyclosporine, four healing completely.
Treatment of Skin Disease Comprehensive Therapeutic Strategies 4e
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Topical tacrolimus
Topical corticoids
Dapsone
Intralesional corticoids
Minocycline
Nicotine
Topical pimecrolimus
Sodium cromoglycate
Sulfasalazine
Cyclosporine
Systemic corticoids
